在接受氧化碳胺治疗的状细胞疾病儿童中,VWF-ADAMTS13轴功能障碍与输血相比
Helen Fogarty1,2,3, Azaz Ahmad1, Ferdows Atiq1
1Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland.
Blood advances
|September 29, 2023
概括
状细胞病 (SCD) 患者的VWF水平升高,特别是那些服用氧化碳胺的患者. 输血治疗可以通过更好地管理VWF-ADAMTS13轴功能障碍和减少血液溶解来改善结果.
科学领域:
- 血液学 血液学 血液学
- 血管生物学 血管生物学
- 儿科医学 儿科医学
背景情况:
- 升高的·威勒布兰德因子 (VWF) 和VWF-ADAMTS13轴功能障碍与状细胞病 (SCD) 血管封闭有关.
- 输血是SCD中风预防的标准治疗方法,但与氧化碳胺相比,其作用机制尚未完全阐明.
研究的目的:
- 为了研究VWF-ADAMTS13轴在SCD患者中接受氧化碳胺与输血的作用.
- 为了比较治疗组之间的内皮激活标志物和血液溶解水平.
主要方法:
- 在180名患有SCD的儿童中测量了血VWF,VWF,第八因子 (FVIII),血管蛋白-2,骨质保护素和血液溶解标志物.
- 患者根据治疗进行了分层:氧化碳胺 (n=96) 或输血 (n=84).
主要成果:
- 尽管接受了治疗,但显著比例的SCD患者表现出VWF和VWF的升高.
- 与输血组相比,氧化碳胺组的VWF参数,FVIII活性和血栓生成显著高于输血组.
- 血液溶解标记与VWF水平有很强的相关性,并且在输血队列中减少.
结论:
- 持续的VWF-ADAMTS13轴功能障碍发生在儿科SCD患者的大量亚组中,特别是那些接受基胺治疗的人.
- 输血可以通过更有效地调节VWF-ADAMTS13轴功能障碍和减少血液溶解来提供卓越的临床益处,而不是氧化碳胺.
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